Sleep deprivation and melatonin for inducing sleep in paediatric electroencephalography: a prospective multicentre service evaluation.

AIM: To compare the efficacy of the main methodologies in attaining sleep and electroencephalography (EEG) abnormalities in children with a view to producing recommendations on best practice. METHOD: Fifty-one UK centres participated. Methods for sleep induction (sleep deprivation, melatonin, and combined sleep deprivation/melatonin) were compared. Data pertaining to demographics, achievement of stage II sleep, and recording characteristics (duration of study, presence of epileptiform activity in awake/sleep states) were prospectively collected for consecutive patients in November and December 2013. RESULTS: Five hundred and sixty-five patients were included. Age range was 1 years to 17 years (mean 7y 10mo), 27.7 per cent had an underlying neurobehavioural condition. Stage II sleep was achieved in 69 per cent of sleep deprived studies, 77 per cent of melatonin studies, and 90 per cent of combined intervention studies (p<0.001, χ2 ). In children who slept, there was no difference between the three interventions in eliciting epileptiform discharges. In children who did not sleep, epileptiform abnormalities were seen more often than after sleep deprivation alone (p=0.02, χ2 ). Seizures were rare. INTERPRETATION: Combined sleep deprivation/melatonin is more effective than either method alone in achieving sleep. The occurrence of epileptiform activity during sleep is broadly similar across the three groups. We recommend the combined intervention to induce sleep for paediatric EEG. WHAT THIS PAPER ADDS: Combined sleep deprivation/melatonin is more effective in achieving sleep than either sleep deprivation or melatonin alone. Sleep latency is shorter with combined sleep deprivation/melatonin. When children do sleep, there is no difference in the occurrence of epileptiform abnormalities between different induction methods. Seizures are rare in sleep electroencephalography recordings.

Home video telemetry in children: A comparison to inpatient video telemetry.

PURPOSE: Home Video Telemetry (HVT) combines ambulatory EEG with simultaneous video recording. No previous reports have compared HVT and inpatient video telemetry (IVT) in a purely paediatric population. This study compares HVT and IVT in this group in terms of diagnostic efficacy, recording quality and acceptability to parents/carers. METHODS: 33 HVT and 29 IVT patients aged 1-17 years were included. Information regarding patient demographics, ictal capture, diagnostic utility, recording quality (e.g. video clarity, EEG artefacts) and parent/carer preferences was documented. Difficulties using HVT equipment were recorded. RESULTS: 62% of IVT patients and 64% of HVT patients had typical attacks during the recording. 59% of IVT and 70% of HVT recordings were considered to have answered the referral question. Study quality was similar in both groups. In HVT studies the rate of equipment difficulties was 52%; problems included camera positioning and failure to turn on the infrared button at night. Diagnostic information was lost in 15% of patients. 76% of parents/carers of HVT patients would choose this investigation again. CONCLUSIONS: The diagnostic efficacy and study quality of HVT and IVT are similar in paediatric patients. HVT is acceptable to most parents/carers. User error may compromise the investigation in a minority of cases but did not impact on diagnostic utility. Adoption of HVT investigation could provide an accessible and economic alternative to IVT.

Optimising the use of EEG in non-epileptic attack disorder: Results of a UK national service evaluation.

PURPOSE: To produce an evidence base to formulate guidelines for optimal performance of EEG in patients referred with a possible diagnosis of non-epileptic attack disorder (NEAD). METHODS: 51 UK EEG departments participated in the prospective study. A pro-forma was completed for all consecutive patients aged 5 years and over referred for EEG over a six month period. Information obtained included referral diagnosis, occurrence/type of attack during EEG, the use of suggestion, length of recording and who was present during the EEG. RESULTS: 11,298 patients were entered into the study. 376 psychogenic non-epileptic seizures (PNES) occurred of which 337 were considered to be of the habitual type. In those patients suspected of having NEAD prior to referral, the use of verbal suggestion increased the yield of habitual attacks by a factor of three in both adults and children. Using suggestive techniques twice, improved the yield further. Non-habitual attacks occurred equally whether or not suggestion was used. At least 90% of habitual PNES occurred within the first 30 min of recording even in those patients having prolonged EEGs. In EEGs where additional professional personnel were present, PNES occurred more frequently. CONCLUSION: This large multicentre study provides evidence to inform recommendations for EEG to investigate NEAD. We recommend the use of verbal suggestion at least twice and where practical the presence of additional professional staff. A thirty minute recording is sufficient to record a habitual PNES in most instances.

Current practice and recommendations in UK epilepsy monitoring units. Report of a national survey and workshop.

PURPOSE: Inpatient video-EEG monitoring (VEM) is an important investigation in patients with seizures or blackouts, and in the pre-surgical workup of patients with epilepsy. There has been an expansion in the number of Epilepsy Monitoring Units (EMU) in the UK offering VEM with a necessary increase in attention on quality and safety. Previous surveys have shown variation across centres on issues including consent and patient monitoring. METHOD: In an effort to bring together healthcare professionals in the UK managing patients on EMU, we conducted an online survey of current VEM practice and held a one-day workshop convened under the auspices of the British Chapter of the ILAE. The survey and workshop aimed to cover all aspects of VEM, including pre-admission, consent procedures, patient safety, drug reduction and reinstatement, seizure management, staffing levels, ictal testing and good data recording practice. RESULTS: This paper reports on the findings of the survey, the workshop presentations and workshop discussions. 32 centres took part in the survey and there were representatives from 22 centres at the workshop. There was variation in protocols, procedures and consent processes between units, and levels of observation of monitored patients. Nevertheless, the workshop discussion found broad areas of agreement on points. CONCLUSION: A survey and workshop of UK epilepsy monitoring units found that some variability in practice is inevitable due to different local arrangements and patient groups under investigation. However, there were areas of clear consensus particularly in relation to consent and patient safety that can be applied to most units and form a basis for setting minimum standards.

Video ambulatory EEG: A good alternative to inpatient video telemetry?

PURPOSE: Video ambulatory EEG (V-AEEG) is a new technique which could add increased capacity for long term EEG monitoring to overstretched inpatient video telemetry (IPVT) services. We compare V-AEEG and IPVT for diagnostic efficacy, recording quality, patient acceptability and technologist time required. METHODS: Forty-one V-AEEG and 64 IPVT adult patients were included. Patients were investigated to diagnose attacks or to obtain polysomnography (PSG) prior to multiple sleep latency test (MSLT). Number of attacks recorded, whether the diagnostic question was answered, quality of video and EEG recording and patients' preference for investigation at home or in hospital were noted. For V-AEEG patients, ease of procedure and extra technologist time required were recorded. RESULTS: Of patients investigated for diagnosis of attacks, 74% V-AEEG patients and 62% IPVT had typical attacks during the investigation. All PSGs were useful in interpreting the MSLTs. Diagnostic questions were answered by 73% V-AEEGs and 73% IPVTs. Quality of EEG and video recording was similar using V-AEEG and IPVT. Four patients had difficulty using V-AEEG equipment but diagnostic information was lost in only one. 5% of V-AEEG patients would have preferred hospital investigation but 45% of IPVT patients would have preferred home investigation. Extra technologist time for home visits (mean 2h) was required only for the first 7 patients. CONCLUSION: Video EEG recording quality and diagnostic efficacy from V-AEEG are similar to IPVT. V-AEEG is acceptable to most patients and does not require additional technical time. Hence, V-AEEG offers a convenient, economical alternative to IPVT.

The role of cranial and thoracic electromyography within diagnostic criteria for amyotrophic lateral sclerosis.

INTRODUCTION: The contribution of cranial and thoracic region electromyography (EMG) to diagnostic criteria for amyotrophic lateral sclerosis (ALS) has not been evaluated. METHODS: Clinical and EMG data from each craniospinal region were retrospectively assessed in 470 patients; 214 had ALS. Changes to diagnostic classification in Awaji-Shima and revised El Escorial criteria after withdrawal of cranial/thoracic EMG data were ascertained. RESULTS: Sensitivity for lower motor neuron involvement in ALS was highest in the cervical/lumbar regions; specificity was highest in cranial/thoracic regions. Cranial EMG contributed to definite/probable Awaji-Shima categorization in 1.4% of patients. Thoracic EMG made no contribution. For revised El Escorial criteria, cranial and thoracic data reclassified 1% and 5% of patients, respectively. CONCLUSION: Cranial EMG data make small contributions to both criteria, whereas thoracic data contribute only to the revised El Escorial criteria. However, cranial and thoracic region abnormalities are specific in ALS. Consideration should be given to allowing greater diagnostic contribution from thoracic EMG. Muscle Nerve 54: 378-385, 2016.

The value of long term EEG monitoring in children: a comparison of ambulatory EEG and video telemetry.

PURPOSE: Outpatient ambulatory EEG may be followed by inpatient video telemetry EEG when investigating children for possible seizures and for classification of epilepsy. We investigated the value of ambulatory EEG and subsequent video telemetry recording in our centre. METHOD: The departmental EEG database was interrogated retrospectively for children undergoing ambulatory recording followed by inpatient video telemetry within an 18-month period. RESULTS: 30 patients fitted these criteria, 21 females, 9 males, age range 3-16 years. The mean interval between studies was 9 months. For ambulatory recordings 93% of studies were undertaken to ascertain if behaviours were epileptic. 66% of ambulatory recordings studies captured an event of interest and 63% were able to answer the question asked of the test. In video telemetry recording 80% of studies were aimed at ascertaining if events were epileptic or not, 20% were undertaken for classification of seizure type. 70% of recordings captured an ictus and were considered helpful in addressing the clinical question. Pooled together 90% of patients had a paroxysmal event captured and the clinical question answered by the recording techniques. In patients for whom ambulatory recording failed to capture an attack or answer the clinical question, 70% went on to have a successful video telemetry recording. CONCLUSION: Both ambulatory EEG and inpatient video telemetry are effective tools for diagnosis of seizures. The majority of patients with failed ambulatory recordings go on to have successful video telemetry. Combining the two resources provides useful clinical information in nearly all instances.

The value of home video with ambulatory EEG: a prospective service review.

PURPOSE: The demand for long term EEG monitoring is increasing with the emphasis on recording patients' attacks. Outpatient ambulatory EEG is relatively inexpensive and widely available. The main disadvantage of the technique is the lack of video which can make interpretation of an ictus difficult. We investigated whether patients, if offered home video equipment, would take it, if this resulted in simultaneous EEG-video capture of an ictus and if interpretation of the recording was facilitated by the video. METHOD: All ambulatory EEG patients, adults and children, were offered a camcorder to take home during a 17-month study period. RESULTS: 130 patients/carers were offered a camcorder (93 adults, 37 children), 45 patients (35%) accepted; the main reason for not accepting was that attacks were considered too brief to record. An ictal event occurred in 34 patients (76%) with a camcorder; in 17 (50%) of these an attack was captured successfully on video. The main reasons for failure to capture events were that attacks were too brief, or that the camcorder was not operated successfully. Attacks were captured with greater success in children (14/23, 61%) than adults (3/11, 27%). Of the 17 video recordings, 14 (82%) were helpful in aiding interpretation of the ambulatory EEG. CONCLUSION: In our study, home video facilities aided interpretation of ambulatory EEG recordings in approximately one third of patients. Technological advances and familiarity with portable recording devices will improve this figure and patients and their carers should be encouraged to use such facilities when available.

The safety of UK video telemetry units: results of a national service evaluation.

PURPOSE: To assess patient safety during seizures occurring on UK video telemetry units and identify factors in unit infrastructure which may improve safety with the intention of producing national guidelines. METHODS: A prospective multicentre national service evaluation of the occurrence of adverse events and level of nurse attendance during seizures occurring on video telemetry units was performed. Data from 272 seizures from 27 video telemetry units across the UK were analysed. RESULTS: Adverse events occurred in 12% of seizures: 7% were physical events such as falls or respiratory compromise and 5% were unnoticed seizures. Nursing staff did not attend the patients in 44% of seizures and attendance was delayed beyond 30s in a further 29%. Only 27% of seizures were attended by a Healthcare Professional within half a minute. The most important factor shown to improve timely attendance of patients during seizures was the presence of a nurse dedicated to the telemetry bed(s). The site of the telemetry bed (bay or cubicle) and method of observation (direct or indirect) was less important. An optimal nurse-to-patient ratio was difficult to identify but the study suggests that a ratio of at least 1 nurse to 4 patients is appropriate. CONCLUSION: The results provide an evidence base for the production of national standards and guidelines for surveillance of patients during video telemetry to improve patient safety.

Patients' and neurologists' perception of epilepsy and psychogenic nonepileptic seizures.

PURPOSE: Although differences in illness perceptions between neurologists and patients with epilepsy or psychogenic nonepileptic seizures (PNES) are likely to be clinically relevant, this is the first study to attempt a direct comparison. In addition, this study compares the illness perceptions of patients with epilepsy with those of patients with PNES. METHODS: Thirty-four patients with epilepsy, 40 patients with PNES, and 45 neurologists were recruited. All patient participants completed versions of the illness perception questionnaire revised (IPQ-R) adapted for epileptic or nonepileptic seizure disorders, single-item symptom attribution question (SAQ), Hospital Anxiety and Depression Scale (HADS), Quality of Life in Epilepsy-31 (QOLIE-31), and Liverpool Seizure Severity Scale (LSSS). Participating neurologists completed two versions of the IPQ-R and two SAQs for epileptic and nonepileptic seizure disorders. KEY FINDINGS: Differences in illness perceptions between patients with epilepsy and patients with PNES were minor compared to those between patients with either seizure disorder and neurologists. Neurologists considered both seizure disorders more treatable and more amenable to personal control than did the patients themselves. Neurologists had much more polarized views of the etiology of both conditions; whereas patients mostly considered the causes of their seizure disorders as partially "physical" and partially "psychological," neurologists perceived epilepsy as an essentially "physical" and PNES as a clearly "psychological" problem. SIGNIFICANCE: There are considerable differences between the illness perceptions of patients with seizure disorders and their doctors, which could represent barriers to successful clinical management. In particular, a discrepancy between neurologists' and patients' beliefs about the personal control that patients may be able to exert over PNES could contribute to the confusion or anger some patients report after the diagnosis has been explained to them. Furthermore, patients' endorsement of "physical" causes for PNES may reflect an unrealistic faith in the effectiveness of "physical" treatments and could be a cause of tension in patients' relationship with their doctor, for instance when the neurologist attempts to withdraw antiepileptic drug treatment or refers patients for psychological interventions.

The natural history of motor neuron disease: assessing the impact of specialist care.

Many centres in the UK care for patients with motor neuron disease (MND) in a multidisciplinary clinic (MDC). It has been demonstrated that such care results in better prognosis for survival than care from a general neurology clinic (GNC). Whether this is due to higher use of disease-modifying interventions or an independent factor of attendance at a specialist clinic has not been established. Hence, we performed a retrospective review of hospital notes of patients with MND who were diagnosed and followed up in a GNC between 1998 and 2002 and in an MDC between 2006 and 2010. Overall, 162 patients attended a GNC, and 255 attended the MDC. The median survival from diagnosis was 19 months for patients who attended the MDC, compared to 11 months for those attending the GNC (hazard ratio 0.51, 95% CI 0.41-0.64). The Cox hazards model identified attendance at an MDC as an independently positive prognostic factor (HR 1.93, 95% CI 1.37-2.72, p < 0.001). We concluded that care at an MDC improves survival. While this effect is augmented by the increased use of riluzole, NIV and PEG, the data suggest that coordinated care independently improves the prognosis of MND patients.

Outcome of children with hyperventilation-induced high-amplitude rhythmic slow activity with altered awareness.

Hyperventilation-induced high-amplitude rhythmic slow activity with altered awareness (HIHARS) is increasingly being identified in children and is thought to be an age-related non-epileptic electrographic phenomenon. We retrospectively investigated the clinical outcome in 15 children (six males, nine females) with HIHARS (mean age 7y, SD 1y 11mo; range 4y 6mo-11y). The presenting feature in 11 cases was blank spells - two of these children also had generalized tonic-clonic seizures (GTCS) - and in one individual the main concern was deteriorating school performance. Three children had symptoms suggestive of focal motor seizures. Of the nine children presenting solely with blank spells, further follow-up (mean duration 18mo, SD 21mo) revealed full resolution of symptoms in six, but three had persistent symptoms. In our study, the symptoms of children with HIHARS presenting with blank spells in isolation appeared to resolve spontaneously and did not evolve into convulsive seizures or other paroxysmal events considered to be clearly epileptic. Children (with HIHARS) who presented with clinical features suggestive of GTCS or focal motor seizures (with or without blank spells) and/or had epileptiform discharges on interictal electroencephalography were subsequently diagnosed with epilepsy.

Illness perceptions of health care workers in relation to epileptic and psychogenic nonepileptic seizures.

Illness perceptions of health care professionals are likely to affect patient care. This study describes the illness perceptions of two groups of health care staff toward epilepsy and psychogenic nonepileptic seizures (PNES). Sixty-one health care professionals (30 emergency care [EC] and 31 neuroscience ward [NW] staff) who regularly see patients with seizures completed the adapted Illness Perception Questionnaire-Revised (IPQ-R) and the Symptom Attribution Question for epilepsy and PNES. Respondents reported a poorer understanding of PNES than of epilepsy (P<0.001), thought epilepsy was a more chronic condition (P=0.001/P<0.001) and that patients with PNES had more "personal control" of their seizures (P=0.014/P<0.001). Staff from both departments identified psychological causes as most important for PNES (P<0.001). EC staff also attributed PNES to behavioral issues or alcohol. The Illness Perception Questionnaire-Revised and Symptom Attribution Question demonstrated important differences in attitudes of health care staff toward epilepsy and PNES. The findings illustrate why some patients with PNES have traumatic encounters with health care professionals.

Stretch syncope: reflex vasodepressor faints easily mistaken for epilepsy.

The pathophysiology of stretch syncope is demonstrated through the clinical, electrophysiological, and hemodynamic findings in three patients. Fifty-seven attacks were captured by video/EEG monitoring. Simultaneous EEG, transcranial (middle cerebral artery) doppler, and continuous arterial pressure measurements were obtained for at least one typical attack of each patient. They all experienced a compulsion to precipitate their attacks. Episodes started with a stereotyped phase of stretching associated with neck torsion and breath holding, followed by a variable degree of loss of consciousness and asymmetric, recurrent facial and upper limb jerks in the more prolonged episodes. Significant sinus tachycardia coincided with the phase of stretching and was followed within 9-16 seconds by rhythmic generalized slow wave abnormalities on the EEG in attacks with impairment of consciousness. Transcranial doppler studies showed a dramatic drop in cerebral perfusion in the middle cerebral arteries during the episodes. The combination of the stereotyped semiology of the attacks, the pseudofocal myoclonic jerking, and the rhythmic generalized slow wave EEG abnormalities with the tachycardia make differential diagnosis from epilepsy challenging.

Clinical review: Sleep measurement in critical care patients: research and clinical implications.

UNLABELLED: Sleep disturbances are common in critically ill patients and have been characterised by numerous studies using polysomnography. Issues regarding patient populations, monitoring duration and timing (nocturnal versus continuous), as well as practical problems encountered in critical care studies using polysomnography are considered with regard to future interventional studies on sleep. Polysomnography is the gold standard in objectively measuring the quality and quantity of sleep. However, it is difficult to undertake, particularly in patients recovering from critical illness in an acute-care area. Therefore, other objective (actigraphy and bispectral index) and subjective (nurse or patient assessment) methods have been used in other critical care studies. Each of these techniques has its own particular advantages and disadvantages. We use data from an interventional study to compare agreement between four of these alternative techniques in the measurement of nocturnal sleep quantity. Recommendations for further developments in sleep monitoring techniques for research and clinical application are made. Also, methodological problems in studies validating various sleep measurement techniques are explored. TRIAL REGISTRATION: Current Controlled Trials ISRCTN47578325.

Myopathy associated with gluten sensitivity.

Ataxia and peripheral neuropathy are the most common neurological manifestations of gluten sensitivity. Myopathy is a less common and poorly characterized additional neurological manifestation of gluten sensitivity. We present our experience with 13 patients who presented with symptoms and signs suggestive of a myopathy and in whom investigation led to the diagnosis of gluten sensitivity. Three of these patients had a neuropathy with or without ataxia in addition to the myopathy. The mean age at onset of the myopathic symptoms was 54 years. Ten patients had neurophysiological evidence of myopathy. Inflammatory myopathy was the most common finding on neuropathological examination. One patient had basophilic rimmed vacuoles suggestive of inclusion-body myositis. Six patients received immunosuppressive treatment in addition to starting on a gluten-free diet; five improved and one remained unchanged. Among seven patients not on immunosuppressive treatment, four showed clinical improvement of the myopathy with a gluten-free diet. The improvement was also associated with reduction or normalization of serum creatine kinase level. The myopathy progressed in one patient who refused the gluten-free diet. Myopathy may be another manifestation of gluten sensitivity and is likely to have an immune-mediated pathogenesis. A gluten-free diet may be a useful therapeutic intervention.

Dietary treatment of gluten neuropathy.

We studied the effect of a gluten-free diet in patients with idiopathic sensorimotor axonal neuropathy and circulating antigliadin antibodies. Consecutive patients underwent baseline neurophysiological assessment and were offered a gluten-free diet. Those who went on the diet formed the intention-to-treat group and those who did not were the control group. Repeat neurophysiological assessment and subjective evaluation of neuropathy symptoms were performed at 1 year. A total of 35 patients participated in the study, with 25 patients going on the diet and 10 not doing so. There was a significant difference in the change of sural sensory action potentials (pre-defined primary endpoint), with evidence of improvement in the intention-to-treat group and deterioration in the control group. Subjective change in neuropathy symptoms also showed significant differences, with patients in the intention-to-treat group reporting improvement and those in the control group reporting deterioration. Gluten-free diet may thus be a useful therapeutic intervention for patients with gluten neuropathy.